Mean Systemic Lupus Erythematosus Disease Activity Index Research Articles

Caracterización de pacientes con lupus y fiebre: actividad, infección o ambas

ObjectiveTo describe clinical characteristics and laboratory findings of patients older than 15years of age diagnosed with Systemic Lupus Erythematosus (SLE) that were admitted to hospital with fever and with a final diagnosis of infection, disease flare-up, or both. MethodsA retrospective chart review of medical charts was performed to identify patients with a diagnosis of SLE with a fever, admitted to the Emergency Department of Hospital Universitario Clinica San Rafael. The data collected included demographics, SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) scores, clinical symptoms, laboratory findings, and steroid use. Results analysis was based on three groups (according to final diagnosis at discharge): patients diagnosed with SLE and infection, SLE and disease flare-up, and SLE with both infection and disease flare-up. ResultsThis study included 108 patients with a mean age of 36, of whom 86% were female. The mean SLEDAI score of all patients analysed was 6 (R 1-15), and for patients with SLE with both infection and disease flare-up was 9.5 (R 6-15). No significant differences were found with clinical symptoms and laboratory findings for any group analysed. SLE patients diagnosed with infections took significantly more prednisolone in the last three months before admission (P=.001). No differences were found with other immunosuppressants. ConclusionThe use of steroids in the last three months, the SLEDAI score, and time since a SLE diagnosis may be variables used to distinguish between infection and disease flare-up in SLE patients presenting with fever. Clinical symptoms and laboratory results are not useful markers to make this distinction.

Comparison of Disease Characteristics, Course and Outcome between Adults with Juvenile and Adult- onset Systemic Lupus Erythematosus

Abstract Aim of the Work This study was performed to evaluate disease characteristics, activity and damage in adult patients with either juvenile or adult onset SLE. Patients and Method One hundred and fifty consecutive patients with SLE fulfilling the SLICC criteria for the classification of SLE were included in the study. The patients were classified according to the age at disease onset, 100 Adult-onset (ASLE) and 50 juvenile onset (JSLE). Patients records were reviewed for clinical and laboratory evaluation. Disease activity was assessed using SLE Disease Activity Index (SLEDAI). Disease damage was assessed using Systemic Lupus International Collaborating Clinics (SLICC/ACR) damage index. Results The mean age at disease onset of JSLE was 14.120± 1.534 and of ASLE was 22.460± 2.732. The female to male ratio was 9:1 in JSLE compared to 13:1 in ASLE. JSLE had butterfly rash (66%), photosensitivity (30%), oral ulcers (34%), arthritis (44%) and neuropsychiatric manifestations (6%).While ASLE had butterfly rash (35%), photosensitivity (10%), oral ulcers (17%), arthritis (65%) and neuropsychiatric manifestations (13%). There was higher prevalence of anemia, thrombocytopenia, ESR, CRP, 24 h and creatinine in JSLE more than ASLE with statistically significant differences. The mean SLEDAI was significantly higher in JSLE (14.080 ± 4.780) compared to ASLE, (10.760 ± 2.738) (P < 0.001). Conclusion The age at onset of SLE influences its clinical manifestations and laboratory findings. JSLE patients have a more aggressive presentation and course than ASLE patients with a higher frequency of lupus nephritis, more cutaneous symptoms and hematological manifestations. JSLE patients have more severe disease activity with very high SLEDAI and SLICC damage scores. ASLE patients have more musculoskeletal manifestations.

Evaluation of Left Ventricular Function in Patients with Systemic Lupus Erythematosus: Association of Tissue Doppler and Strain Imaging with Disease Activity

Background: Systemic lupus erythematosus (SLE) is a connective tissue disorder with cardiovascular involvement associated with high morbidity and mortality. Routine echocardiography often misses early myocardial involvement. We intend to use tissue Doppler imaging (TDI), strain and strain rate imaging to reveal subclinical myocardial dysfunction in asymptomatic females with SLE; and its correlation with disease activity and anticardiolipin antibodies. Materials and Methods: Forty-three female SLE patients without cardiac symptoms or signs and matched healthy control group (n = 20) underwent standard echocardiography, TDI, strain and strain rate imaging. Disease activity of SLE was assessed using the SLE disease activity index (SLEDAI); ≥6 points were considered active. Results: Mean age of SLE patients was 29.86 years with a mean SLEDAI score of 4.36 ± 4.5. Standard two dimensional-Echocardiogram parameters were similar to healthy controls. SLE was associated with significantly impaired systolic myocardial velocities of left ventricle measured by TDI; medial S': 8.5 ± 1.2 versus 9.6 ± 1.0 cm/s, P = 0.007; lateral S': 9.2 ± 1.7 versus 11.4 ± 1.6 cm/s, P = 0.012); and decrease in strain (−17.2% ± 2.2% vs. −20.95% ± 2.1%; P

Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus: Experiences From a Chinese Tertiary Medical Center.

ObjectiveBecause acquired hemophilia (AH) is a rare entity in systemic lupus erythematosus (SLE), we aimed to investigate the clinical features of SLE-related AH in Chinese patients.MethodsThis is a medical records review study carried out at a large tertiary care hospital in China from years 1986 to 2018. We searched the case database in Peking Union Medical College Hospital using the International Classification of Diseases. The clinical data on SLE-related AH patients were collected.ResultsA total of 9282 SLE patients had been hospitalized. Six female SLE-related AH patients were identified. Four patients had acquired hemophilia A (AHA), and 2 patients had acquired von Willebrand syndrome. Their mean age was 33.67 ± 13.77 years. Five patients had active disease. The mean SLE disease activity index measured at the time of diagnosis of AH was 10.50 ± 5.28. The average level of activated partial thromboplastin time was 86.5 seconds. Coexistence of secondary antiphospholipid syndrome and AHA was found in one case, and pulmonary embolism was observed 3 years later. After immunosuppressive therapy and symptomatic treatment, an overall remission rate of 83.3% was achieved.ConclusionsThe frequency of SLE-related AH was low. The development of AH in SLE patients frequently occurs with active disease. The AH could be the first clinical presentation of SLE. Secondary antiphospholipid syndrome and AHA could appear in the same SLE patient. Early and aggressive treatment contributes to a favorable prognosis.

Identifying damage clusters in patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) causes irreversible damage to organ systems. Recently, evidence has been obtained for subphenotypes of SLE. This study aimed to identify damage clusters and compare the associated clinical manifestations, SLE disease activity, mortality, and genetic risk scores (GRS). The study was conducted on the Hanyang BAE lupus cohort. Patients with disease duration <5years were excluded to minimize confounding effects of disease duration. They were grouped into 3 clusters based on the Systemic Lupus International Collaborating Clinics Damage Index using k-means cluster analysis. Among the 1130 analyzed patients, musculoskeletal damage was most prevalent (20.2%), followed by ocular (11.4%), renal (10.5%), and neuropsychiatric damage (10.2%). Three significantly different damage clusters were identified. Patients in cluster 1 (n=824) showed the least damage. Cluster 2 (n=195) was characterized by frequent renal (55.4%) and ocular (58.0%) damage, and cluster 3 (n=111) was dominated by neuropsychiatric (100%) and musculoskeletal damage (35.1%). Cluster 2 had the highest adjusted mean AMS (adjusted mean SLE Disease Activity Index score; mean±SD: 5.4±2.9), while cluster 3 had the highest mortality (14.4%). Weighted GRS did not differ significantly between the clusters. Patients in prevalent renal and ocular damage cluster had the highest AMS scores, while the cluster with frequent neuropsychiatric damage had the highest mortality.

Risk factors for cataracts in a cohort of Egyptian systemic lupus erythematosus patients

Aim of the workThis study was undertaken to evaluate cataract risk factors in Egyptian systemic lupus erythematosus (SLE) patients. Patients and methodsA cohort of 150 consecutive adult SLE patients was included. None of the patients was diabetic or had cataract before steroids treatment for SLE. Detailed medical and ophthalmological histories were taken. Thorough clinical examination was performed with assessment of SLE disease activity index (SLEDAI) and systemic lupus international collaborating clinics damage index (SLICC DI), along with routine blood tests. Slit lamp examination was done to assess the anterior segment of the eye especially the lens to detect the type and degree of cataract. Results150 SLE patients were studied with a mean age of 37.16 ± 11 years; 129 females and 21 males and a mean disease duration of 11 ± 5.7 years. Their mean SLEDAI was 4.1 ± 3.78 and SLICC/DI 2.1 ± 1.3. The frequency of cataract was 18% (n = 27); 22.2% of them were cortical and 77.8% were posterior subcapsular with mild-moderate degree. Glaucoma was present in only 2%. A significant increase in age, disease duration, age at onset, SLICC/DI, cumulative steroid dose and hydroxychloroquine (HCQ) dose was found in those with cataract compared to non-cataract patients (p < 0.05). The age, age at onset, disease duration, SLICC/DI and cumulative steroid dose were significant independent factors increasing the probability of cataract occurrence among SLE patients (p < 0.01). ConclusionAlthough corticosteroids are prescribed to control SLE symptoms, these results highlight potential risks associated with their use, especially cataracts, which require careful and routine ophthalmologic examination and follow up.

Juvenile systemic lupus erythematosus: a single-center experience from southern Turkey.

This study was conducted to analyze clinical characteristics, laboratory data, disease activity, and outcome of juvenile systemic lupus erythematosus (jSLE) patients from southern Turkey. Fifty-three patients with jSLE diagnosed according to the revised American College of Rheumatology 1997 criteria between January 2005 and June 2018 were included in the present study. The median age at the diagnosis was 12.8 (range, 5.1-17.7) years. The female to male ratio was 9.6:1. The most prevalent clinical features were mucocutaneous involvement (96.2%) and constitutional manifestations (94.3%). Renal manifestations, hematological manifestations, and neuropsychiatric involvement were detected in 40 (75%), in 38 (71.7%), and in 13 (24.5%) patients, respectively. Renal biopsy was performed to 49 patients (92.5%). Class IV lupus nephritis (LN) (34%) and class II LN (20.4%) were the most common findings. Mycophenolate mofetil, cyclophosphamide with corticosteroid were the main treatment options. Eighteen patients received rituximab and one tocilizumab. The mean SLE Disease Activity Index (SLEDAI) score at the time of diagnosis was 22.47 ± 8.8 (range = 3-49), and 1.34 ± 1.85 (range = 0-7) at last visit. Twenty-one patients (39.6%) had damage in agreement with Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (PedSDI; mean = 0.60 ± 0.94; range = 0-5) criteria. Growth failure was the most prevalent cause of damage (n = 13, 26%). One patient deceased due to severe pulmonary hemorrhage and multiple cerebral thromboses. jSLE patients in this cohort have severe disease in view of the higher frequency of renal and neurologic involvement. Nevertheless, multicenter studies are needed to make a conclusion for all Turkish children with jSLE.

Mortality in systemic lupus erythematosus at a teaching hospital in India: A 5-year retrospective study.

Objective:Systemic lupus erythematosus (SLE) is an autoimmune disease with an unknown etiology that can be life threatening. This study aimed to study the cause of mortality among admitted SLE patients over a period of 5 years at a teaching hospital in India.Methods:A 5-year retrospective analysis of mortality in SLE patients admitted under department of medicine of our institute was done. The presenting complaints, treatment history, clinical parameters, laboratory investigations, organ involvement, systemic lupus erythematosus disease activity index (SLEDAI), and cause of mortality were collected from the medical records on a predesigned proforma. A further analysis of two groups based on the cause of mortality was done.Results:In total, 53 death records were analyzed. Mortality in 28 SLE patients was due to high disease activity (Group I) and mortality in 25 patients was attributed due to both high disease activity and concomitant infection (Group II). Most of the patients were female (98%) and mean age of patient was 30.6 years. About 19 patients (35.8%) were diagnosed with SLE during hospital admission. Fever was the most common presenting complaint (69.8%) and lupus nephritis was the most common organ dysfunction seen (84.9%). Myocarditis was observed in 11 patients and 9 patients had cerebrovascular accident. The mean hemoglobin was lower in Group II (7.4 vs. 8.7 g/dL, P = 0.02). The median total leukocyte count was significantly higher in Group II (10,200 vs. 6600, P = 0.02). The mean serum urea and creatinine levels were also significantly higher in Group II (141.41 vs. 87.8 mg/dL, P = 0.006 and 4.7 vs. 1.7, P = 0.0001), respectively. The mean SLEDAI in Group I was 20.8 ± 8.9 and in Group II was17.7 ± 7.5. Bacterial pneumonia (17) was the most common infection, followed by tuberculosis (2) and fungal infection (2).Conclusion:Mortality among SLE patients could be due to disease flare or concomitant infection. Lung is the most common organ affected by infection in these patients.

Diagnostic potential of metastasis-associated-lung-adenocarcinoma-transcript-1 (MALAT-1) and TNFα and hnRNPL related immunoregulatory long non-coding RNA (THRIL) in systemic lupus erythematosus patients: Relation to disease activity

Aim of the workTo determine expression levels and diagnostic value of metastasis-associated-lung-adenocarcinoma-transcript-1 (MALAT-1) and TNFα and hnRNPL related immunoregulatory long non-coding RNA (THRIL) in systemic lupus erythematosus (SLE), and to assess their role in the clinical characteristics of SLE and disease activity. Patients and methodsStudy included 40 patients with SLE and 30 matched controls. SLE Disease Activity Index (SLEDAI) score was assessed. Expression levels of MALAT-1 and THRIL were detected in the serum by using Real-time polymerase chain reaction and 2−ΔΔCT method. ResultsMean age of patients was 40.1 ± 9 years (25–55 years), they were 38 females and 2 males and disease duration was 16.5 ± 3.9 years. Their mean SLEDAI was 5.8 ± 5.3. Expression levels of MALAT-1 and THRIL were found to be significantly upregulated in the serum of SLE patients compared with controls (set as 1). MALAT-1 fold change = 3.7 ± 3.8 (p = 0.009), and THRIL fold change = 3.6 ± 3.4 (p = 0.026). There were significant correlations between MALAT-1 with THRILL (r = 0.44, p = 0.005), proteinuria (r = 0.45, p = 0.006), erythrocyte sedimentation rate (r = 0.43, p = 0.006) and SLEDAI (r = 0.36, p = 0.024). No significant correlations were found between THRIL and study parameters. Sensitivity and specificity of MALAT-1 and THRIL were determined (sensitivity 67.5% and 65% respectively), (specificity 100% for both, total accuracy 80% and 81.4% respectively), and the combined effect of both increased sensitivity and total accuracy to 70% and 82.9% respectively. THRIL was a significant predictor for SLE disease (p = 0.02). ConclusionMALAT-1 and THRIL may be potential diagnostic biomarkers for SLE and only MALAT-1 may be valuable in detecting disease activity.

Association of systemic lupus erythematosus activity with serum levelsof sTWEAK and CD160: A cross-sectional study

Introduction: Systemic lupus erythematosus (SLE) is a relatively common disease among the patients referred to the rheumatology clinics. Tumor necrosis factor (TNF)-like weak inducer of apoptosis (TWEAK), as a cytokine, is a member of TNF family, and CD160 is an essential natural killer (NK) cell activator, both of which have been argued to be associated with SLE activity. Here, we aimed to evaluate the serum levels of sTWEAK and CD160 and their association with SLE activity. Methods: In a descriptive cross-sectional study, 48 patients with SLE, as the case group, and 40 healthy subjects, as controls, were enrolled. SLE activity was assessed using SLE Disease Activity Index (SLEDAI) in the case group. Moreover, the serum levels of sTWEAK and CD160 were determined using enzyme linked immunosorbent assays (ELISA) method in both groups. Results: Mean serum level of sTWEAK was 19.09% lower in the control group compared to the case group (730.15 ± 170.21 pg/ml vs. 895.39 ± 451.25 pg/ml, respectively). Further, mean serum level of CD160 was 47.31% lower in the healthy subjects than that of SLE patients (206.16 ± 88.97 pg/ml vs 391.30 ± 283.46 pg/ml, respectively). The differences in both occasions were found to be significant (P = 0.013 and P =0.001, respectively). Mean SLEDAI in the patients was 8.68 ± 4.00. There was no significant correlation between serum levels of sTWEAK and CD160 with SLE activity. Conclusion: The serum levels of sTWEAK and CD160 markers in patients with SLE are significantly higher than those of healthy subjects. However, we found no correlation of these markers with the disease activity.

Investigation of MicroRNA in Mitochondrial Apoptotic Pathway in Systemic Lupus Erythematosus

Background Accumulating evidence indicates that microRNAs play a pivotal role in the pathogenesis of systemic lupus erythematosus (SLE). This study tested the hypothesis that microRNA is associated with the mitochondrial apoptotic pathway in patients with SLE. Methods Thirteen patients were in the clinical comparison study and microRNA study and overall 19 patients in the study of intracellular protein. Levels of microRNAs were determined by miRNeasy kit in 13 patients with SLE and 29 volunteer normal controls. Intracellular levels of caspase-9, caspase-10, MAVS, MDA5, and pIRF7 in mononuclear cells from 19 patiens and the SLE disease activity index (SLEDAI) were determined in all SLE patients. Correlation analyses were performed among microRNAs, intracellular adaptor proteins, and caspase levels and mean SLEDAI. Results The ΔCT, defined by test reading difference between the target and the internal control microRNA (miR-451a), of miR-21-5p, miR-150-5p, and miR221-3p were significantly higher in plasma from SLE patients than in normal controls. miR-150-5pΔCT was positively correlated with both CRP and SLEDAI value. miR-150-5pΔCT was negatively associated with MAVS 70 kD. Caspase-10 protein levels were negatively associated with plasma miR-22-3pΔCT and miR-21-5pΔCT levels. Conclusions Our study confirmed the hypothesis that these microRNAs were associated with the mitochondrial apoptotic pathway in SLE. miR-150-5pΔCT was positively associated with SLE disease activity and it was negatively correlated with MAVS 70 kD, which may facilitate viral survival and further enhance inflammation. On the other hand, miR-22-3pΔCT and miR-21-5pΔCT, were negatively correlated with caspase-10 levels, which may repress extrinsic apoptosis and increase cell survival.

Sirolimus in patients with clinically active systemic lupus erythematosus resistant to, or intolerant of, conventional medications: a single-arm, open-label, phase 1/2 trial

Patients with systemic lupus erythematosus have T-cell dysfunction that has been attributed to the activation of the mammalian target of rapamycin (mTOR). Rapamycin inhibits antigen-induced T-cell proliferation and has been developed as a medication under the generic designation of sirolimus. We assessed safety, tolerance, and efficacy of sirolimus in a prospective, biomarker-driven, open-label clinical trial. We did a single-arm, open-label, phase 1/2 trial of sirolimus in patients with active systemic lupus erythematosus disease unresponsive to, or intolerant of, conventional medications at the State University of New York Upstate Medical University (Syracuse, NY, USA). Eligible participants (aged ≥18 years) had active systemic lupus erythematosus fulfilling four or more of 11 diagnostic criteria defined by the American College of Rheumatology. We excluded patients with allergy or intolerance to sirolimus, patients with life-threatening manifestations of systemic lupus erythematosus, proteinuria, a urine protein to creatinine ratio higher than 0·5, anaemia, leucopenia, or thrombocytopenia. Patients received oral sirolimus at a starting dose of 2 mg per day, with dose adjusted according to tolerance and to maintain a therapeutic range of 6-15 ng/mL. Patients were treated with sirolimus for 12 months. Safety outcomes included tolerance as assessed by the occurrence of common side-effects. The primary efficacy endpoint was decrease in disease activity, assessed using the British Isles Lupus Assessment Group (BILAG) index and the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Blood samples of 56 matched healthy individuals were obtained as controls for immunobiological outcomes monitored at each visit. The primary efficacy endpoint was assessed in all patients who completed 12 months of treatment, and all patients who received at least one dose of treatment were included in the safety analyses. This trial is registered with ClinicalTrials.gov, number NCT00779194. Between March 9, 2009, and Dec 8, 2014, 43 patients were enrolled, three of whom did not meet eligibility criteria. 11 of the 40 eligible patients discontinued study treatment because of intolerance (n=2) or non-compliance (n=9). SLEDAI and BILAG disease activity scores were reduced during 12 months of treatment in 16 (55%) of 29 patients who completed treatment. Mean SLEDAI score decreased from 10·2 (SD 5·6) at enrolment to 4·8 (4·5) after 12 months of treatment (p<0·001) and the mean total BILAG index score decreased from 28·4 (12·4) at enrolment to 17·4 (10·7) after 12 months of treatment (p<0·001). The mean daily dose of prednisone required to control disease activity decreased from 23·7 mg (SD 9·6) to 7·2 mg (2·3; p<0·001) after 12 months of treatment. Sirolimus expanded CD4+CD25+FoxP3+ regulatory T cells and CD8+ memory T-cell populations and inhibited interleukin-4 and interleukin-17 production by CD4+ and CD4-CD8- double-negative T cells after 12 months. CD8+ memory T cells were selectively expanded in SRI-responders. Patient liver function and lymphocyte counts were unchanged. Although HDL-cholesterol (Z=-2·50, p=0·012), neutrophil counts (Z=-1·92, p=0·054), and haemoglobin (Z=-2·83, p=0·005) were moderately reduced during treatment, all changes occurred within a range that was considered safe. Platelet counts were slightly elevated during treatment (Z=2·06, p=0·0400). These data show that a progressive improvement in disease activity is associated with correction of pro-inflammatory T-cell lineage specification in patients with active systemic lupus erythematosus during 12 months of sirolimus treatment. Follow-up placebo-controlled clinical trials in diverse patient populations are warranted to further define the role of mTOR blockade in treatment of systemic lupus erythematosus. Pfizer, the National Institutes of Health, and the Central New York Community Foundation.

Progranulin and insulin-like Growth Factor Binding Protein-2 as Biomarkers of Disease Activity and Pathological Changes in Lupus Nephritis

Abstract Background Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease, characterised by the production of auto-antibodies and the formation of immune complexes due to the polyclonal activation of T and B lymphocytes, which results in tissue and organ damage. During inflammation, neutrophils and macrophages release serine proteases to cleave progranulin (PGRN) into granulin, which exerts its pro-inflammatory effects that counteract the anti-inflammatory effects of intact PGRN. It is suggested that insulin-like growth factor binding protein-2 (IGFBP-2) is a dependable biomarker of renal deterioration but it is still unclear if it has high sensitivity and specificity for discriminating SLE-caused kidney disease from other-cause kidney disease.This study aimed to investigate the diagnostic value of PGRN and ILGFBP-2 in patients with lupus nephritis (LN) and the correlation of these biomarkers with disease activity and renal biopsy pathology. Patients and methods Patients with SLE (n=25) and chronic kidney disease (CKD) (n=25), and age- and sex-matched controls (n=25) were enrolled in the study. Serum PGRN and ILGFBP-2 levels were measured for each group. Results Disease duration was 4.78±4.26 years in the SLE patients. The mean SLE Disease Activity Index score was 15.04±7.54. All renal biopsy results were class 2, 3, and 5 with a percentage of 32, 24, and 44% respectively. PGRN and ILGFBP-2 were significantly higher in SLE patients (p&lt;0.001 all) than in the CKD and control groups. All patients with high levels of biomarkers showed higher values of SLE disease activity. No significant difference was noted between active and inactive LN or classes of renal biopsy with PGRN and ILGFBP-2. Conclusion PGRN and ILGFBP-2 are significantly elevated in SLE compared to CKD and the general population and were associated with the SLE Disease Activity Index but not with active LN or classes of renal biopsy.

Toward electronic health recording: evaluation of electronic patient reported outcome measures (e-PROMs) system for remote monitoring of early systemic lupus patients.

The study aimed to assess the value of evaluation of electronic patient reported outcome measures (e-PROMs) in the assessment and management of SLE disease activity flares, its association with adherence to therapy as well as organ damage. A randomized, controlled crossover study was carried out over a 24-month duration. One hundred forty-seven SLE patients meeting the revised American College of Rheumatology (ACR) criteria were enrolled. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was used to assess disease activity, whereas organ damage was scored using the Systemic Lupus International Collaborating Clinics (SLICC)/ACR Damage Index. In the first 12months, the patients were assessed every 3 months. At 12months, the patients were randomized into a cohort of 73patients who continued their care in the same style and 74patients who completed an online e-PROMs questionnaire on monthly basis for another 12-month period. The data captured were then retrospectively analyzed at the end of the 24-month study period. At the end of the first year of the study, the mean SLEDAI and SDI scores were 8.72 (6.1) and 1.9 (2.2). At the end of the second year, the mean SLEDAI and SDI scores in the e-PROMs cohort were 3.1 (2.6) and 1.2 (1.3), whereas in the control group, the scores were 7.63 (6.7) and 1.8 (2.3), respectively (p<0.01). Adjusting for possible confounding variables, the number of flares, regardless of their severity, was associated with damage accrual (OR 2.03, 95% CI 1.34 to 2.83, p<0.001). Adherence to therapy was significantly (p<0.1) higher in the e-PROMs group. e-PROMs was equivalent to PROMs paper format and has a potential disease-modifying effect as it facilitated close monitoring of disease activity with an option of management escalation whenever indicated.

CORRELATION BETWEEN ANTI-DSDNA, COMPLEMENT COMPONENTS C3, C4 AND SYSTEMEIC LUPUS ERYTHEMATOSUS DISEASE ACTIVITY INDEX

Background: To find out the correlation between anti-ds DNA, complement components C3, C34 and disease activity in SLE. Material and Methods: This cross-sectional study was undertaken over a period of 6 months from January 2016 to June 2016 at a Tertiary Care Centre in South India. Blood samples were collected from 100 patients with active lupus, 100 patients with inactive disease and 100 healthy controls. Serum samples from all the 300 samples were tested for anti- dsDNA by ELISA and C3, C4 by Radial Immunodiffusion. Results: High titre anti-dsDNA was detected in 48 out of the 100 patients with active disease. Mean SLE disease activity index (SLEDAI) of patients with high titre anti-dsDNA was 23.08337 ± 3.80276 (P<0.001) Mean SLEDAI of patients with very low C3 and C4 was 21.4314 ± 4.88776 (P<0.001) and 21.1250 ± 4.82882 (P<0.001). Conclusion: Anti-dsDNA, C3, C4 levels can be adopted as markers of disease activity in SLE. Anti-dsDNA by ELISA is a simple and cost effective method. Radial immunodiffusion can be done for C3 and C4 assay where nephelometer is not available as the procedure is simple.

Expression of the leukocyte-associated Ig-like receptor-1 on B lymphocytes from systemic lupus erythematosus patients

The leukocyte-associated immunoglobulin (Ig)-like receptor-1 (LAIR-1) is a transmembrane molecule belonging to the Ig superfamily. In B cells, LAIR-1 cross-linking leads to downregulation of Ig and cytokine production. The aim of the present study was to assess the expression of LAIR-1 on peripheral blood B lymphocyte from systemic lupus erythematosus (SLE) patients, and its correlation with disease manifestations. Twenty-two SLE female patients and 16 matched healthy controls were included in the study. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score was assessed. The expression of LAIR-1 was determined by using flow cytometry. The 22 SLE patients had a mean age of 25.59±4.22 years and disease duration of 5–24 months. The mean SLEDAI was 8±1.5. The percentage of CD19+ B lymphocytes lacking LAIR-1 was markedly increased in SLE patients (27.8 ±10.9%) compared with healthy controls (16.2±4.4%) (P<0.001). The mean fluorescent intensity ratio (MFIR) of LAIR-1 expression on CD19+ B cells was strongly decreased in SLE patients (44.1±12.6) compared with healthy controls (58.9±7.7) (P<0.001). The percentage of CD19+ LAIR-1− B cells significantly correlated with the complement (C4) (r=0.45, P=0.03) and the serum creatinine level (r=0.47, P=0.02), and negatively with the serum albumin level (r=−0.57, P=0.005). The MFIR of LAIR-1 significantly correlated with the serum albumin level (r=0.74, P<0.001) and negatively correlated with the serum creatinine level (r=−0.43, P=0.041). There was no significant association of LAIR− (%) or the MFIR with the clinical manifestations of the patients. This study points out that the lack of LAIR-1 expression on B cells from SLE patients could be a trigger for the dysregulation of antibody production in SLE, and is associated with the degree of renal affection as evidenced by the significant correlation with serum creatinine levels and negative correlation with the levels of serum albumin.

Immunological profile and dyslipidemia in Egyptian Systemic Lupus Erythematosus patients

Aim of the workTo study the relation of the immune profile to dyslipidemia in a cohort of Egyptian Systemic Lupus Erythematosus (SLE) patients. Patients and methodsThis study included 221 SLE patients with a disease duration >6months at study entry. Disease activity was assessed using the SLE Disease Activity Index (SLEDAI) and severity using the Systemic Lupus International Collaborating Clinics/Damage Index (SLICC/DI). Patients were investigated for the anti-nuclear antibody (ANA), anti-double stranded deoxy-ribonucleic acid (anti-dsDNA), anti-cardiolipin (ACL) antibodies (IgG and IgM), anti Ro (SSA) and anti La (SSB). Dyslipidemia was considered if the high density lipoproteins (HDL), low density lipoproteins (LDL), total cholesterol (TC) or triglycerides (TG) were abnormal. ResultsThe mean age of the patients was 28.8±7.8years and the median disease duration was 5years. The clinical manifestations of the patients were pleurisy (52.9%), pericarditis (24.9%), nephritis (68.3%), CNS lupus (23.1%), vasculitis (14.9%) and musculoskeletal manifestations (57.9%). All patients were on corticosteroids (median dose 35mg/day; range 5–80mg/day), while 92 (43.4%) of them received cyclophosphamide during their disease course. The mean SLEDAI was 12.1±7.4 and SLICC/DI was 1.4±1.6. Patients with positive anti-Ro (n=44; 19.9%) showed statistically significant lower level of HDL (p=0.01). ConclusionPositive anti-Ro may be associated with increased incidence of low HDL in lupus patients which in turn may increase the incidence of cardiovascular accidents.

Differences in Clinical Features and Mortality between Childhood-onset and Adult-onset Systemic Lupus Erythematosus: A Prospective Single-center Study.

To compare clinical features and mortality between childhood-onset systemic lupus erythematosus (cSLE) and adult-onset SLE (aSLE) in a prospective single-center cohort. A total of 1112 patients with SLE (133 cSLE and 979 aSLE) were enrolled and followed from 1998 to 2012. The 2 groups were compared regarding American College of Rheumatology (ACR) classification criteria for SLE, autoantibodies, disease activity measured by the Adjusted Mean SLE Disease Activity Index (AMS), damage measured by the Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI), and medication. The standardized mortality ratio (SMR) was calculated. Predictors of mortality in SLE were evaluated using Cox proportional hazard models. After a mean followup of 7.6 years, patients with cSLE had a higher number of cumulative ACR criteria and a higher AMS (p < 0.001 each), but there was no difference in SDI (p = 0.797). Immunosuppressants were used more frequently by patients with cSLE (p < 0.001). The SMR of cSLE was 18.8 (95% CI 8.6-35.6), significantly higher than that of aSLE (2.9, 95% CI 2.1-3.9). We found cSLE to be an independent predictor of mortality (HR 3.6, p = 0.008). Moreover, presence of hemolytic anemia (7.2, p = 0.034) and antiphospholipid antibody (aPL; 3.8, p = 0.041) increased the magnitude of risk of early mortality more in the patients with cSLE than in those with aSLE. The clinical course of cSLE as measured by number of clinical manifestations and disease activity is worse than that of aSLE. Also, cSLE patients with hemolytic anemia and aPL are at greater risk of death than patients with aSLE who have those features.

Association between anti-Ro 60 kDa (SS-A) autoantibodies and hypocomplementemia in systemic lupus erythematosus patients from Algiers prefectures

BackgroundSystemic lupus erythematosus (SLE) is characterized by a vicious cycle maintaining systemic inflammation. It starts by autoantibody production, immune complex formation and complement activation that contribute to inflammation, tissue damage and further autoantibody production. Aim of the workTo evaluate the association between the auto-antibodies (abs), circulating immune complexes (CIC), and complement activity in SLE patients. Patients and methodsThis study involved 30 female SLE patients analyzed for autoantibodies, complement profile including complement hemolytic 50 (CH50), alternative pathway 50, factor B, C1q, C2, C3 and C4 as well as C1q-CIC. SLE disease activity was assessed by the SLE Disease Activity Index (SLEDAI). ResultsThe age of patients was 34±12.8years, disease duration was 5.2±3.2years and their mean SLEDAI was 9.96±4.2. Anti-SSA, anti-dsDNA, anti-C1q abs, and CIC were detected in 36.7%, 50%, 50% and 30% of patients, respectively. Anti-SSA were higher in patients with lower compared to normal CH50 activity and C3 level (24.7 vs 88.6U/ml; p=0.002 and 118.6±25.18U/ml vs 15.9±7.3; p<0.0001 respectively) than the other autoantibodies. Increased CIC were higher in patients with lupus nephritis and were associated with anti-SSA, anti-SSB, anti-C1q, anti-Sm and in patients with low CH50 activity. The CIC significantly correlated with anti-C1q (r=0.69, p<0.0001), anti-SSA (r=0.5, p=0.005) and negatively with CH50 (r=−0.4, p=0.046). ConclusionsThe current study confirms that the etiopathogenic anti-SSA autoantibodies are the most associated with hypocomplementemia in SLE. This would stimulate future researches for validation of predictive biomarkers earlier than hypocomplementemia which is still the major unmet need in lupus research and patient care.

Can pregnancy induce relapse in systemic lupus erythematosus (SLE)?

Objective: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease which mostly affects women of reproductive age. We evaluated the impact of pregnancy on maternal/fetal health, the pattern of organ involvements and the fare-up risk. Methods: In a retrospective study we studied the thirty-year medical records of patients between 1976-2005. Maternal, neonatal and infantile health data was retrieved. Incidence of flare-ups, pattern of organ involvements and the outcome of pregnancy was analyzed. Results: We studied 155 pregnancies in 129 SLE patients. Mean age of patients was 27.0 ± 5.5 years (range, 16-44). Thirty one cases (20.2%) experienced flares in the course of pregnancy. During pregnancy, SLE disease activity index (SLEDAI) score increased in 92 (59.3%) patients (median increase = 6 scores). On the other hand, 38 cases (24.5%) SLEDAI score remained unaltered and in 25 cases (16.1%) SLEDAI score decreased (median decrease = 1). Mean SLEDAI during pregnancy were significantly higher than preconceptional scores (P-value = 0.002).Term delivery was more common in quiescent SLE (54.2% vs. 34.6%, P-value = 0.04). Number of therapeutic abortions was higher in active SLE (38.5% vs. 10.2% P-value = 0.003). Conclusion: In this study increased SLEDAI and flare-up episodes were observed during pregnancy. However the majority of cases did not face major fetal or maternal complications.